Pasqualini syndrome E23.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Eunuchism with spermiogenesis; Eunuchs fertile; Fertile eunuch syndrome

History
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Pasqualini and Bur, 1950

Definition
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Isolated deficiency of LH with normal FSH secretion due to incremental testicular dysfunction with secondary Leydig cell insufficiency.

Clinical features
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Eunuchism, large slender appearance without secondary sexual characteristics, almost normal testicle size

Laboratory
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normo- to oligozoospermia ("fertile eunuchs"), LH and total gonadotropin excretion in urine reduced.

Therapy
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With entry into puberty hormone substitution. Initial testosterone cantate (e.g. Testoviron depot 50) i.m. 50 mg/month. Slowly increase dose within 1-2 years up to 250 mg every 2-3 weeks, e.g. testosterone cantate (e.g. Testoviron Depot 250).

Literature
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  1. Pasqualini RQ, Bur EC (1950) Sindrome hipoandrogenico con gametogenesis conservada: clasificacion de la insuficiencis testicular. Rev Assoc Med Argent 64: 6

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020